Unilateral Retinal Dysplasia

Although the term "retinal dysplasia" was used in a general sense by earlier writers in reference to abnormalities in development of the retina,1,2its recent use in many instances reflects the special interpretation placed on it in an article by Reese and Blodi in 1950.3These authors used the term to designate a syndrome consisting of congenital anomalies involving principally the brain, heart, extremities, mouth and eye. They gave the syndrome this name because the dysplastic retina was the only feature common to all of these cases. In each of their eight cases the ocular abnormality was bilateral and occurred in newborn full-term infants. The eyes were usually microphthalmic, and with an ophthalmoscope a whitish mass consisting of persistent primary vitreous and dysplastic retina could be seen behind the lens. In a second article in 1958, Reese and Straatsma presented 17 additional cases of this syndrome, in ten